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Article ; Online: How I Treat Focal Segmental Glomerulosclerosis.

Liew, Adrian / Gibson, Keisha L

Clinical journal of the American Society of Nephrology : CJASN

2022 Volume 17, Issue 12, Page(s) 1814–1816

MeSH term(s)	Humans ; Glomerulosclerosis, Focal Segmental/complications ; Glomerulosclerosis, Focal Segmental/drug therapy ; Kidney Transplantation ; Recurrence
Language	English
Publishing date	2022-08-23
Publishing country	United States
Document type	Journal Article
ZDB-ID	2226665-3
ISSN	1555-905X ; 1555-9041
ISSN (online)	1555-905X
ISSN	1555-9041
DOI	10.2215/CJN.06850622
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Article ; Online: Finding That Needle in the Haystack: Computable Phenotypes.

Glenn, Dorey / Gibson, Keisha L

Journal of the American Society of Nephrology : JASN

2019 Volume 30, Issue 12, Page(s) 2279–2280

MeSH term(s)	Child ; Electronic Health Records ; Humans ; Kidney Diseases ; Phenotype
Language	English
Publishing date	2019-11-15
Publishing country	United States
Document type	Editorial ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.2019101037
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Zs.A 3344: Show issues

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Article ; Online: COL4A gene variants are common in children with hematuria and a family history of kidney disease.

Rheault, Michelle N / McLaughlin, Heather M / Mitchell, Asia / Blake, Lauren E / Devarajan, Prasad / Warady, Bradley A / Gibson, Keisha L / Lieberman, Kenneth V

Pediatric nephrology (Berlin, Germany)

2023 Volume 38, Issue 11, Page(s) 3625–3633

Abstract: Background: Inherited kidney diseases are a common cause of chronic kidney disease (CKD) in children. Identification of a monogenic cause of CKD is more common in children than in adults. This study evaluated the diagnostic yield and phenotypic spectrum ...

Abstract	Background: Inherited kidney diseases are a common cause of chronic kidney disease (CKD) in children. Identification of a monogenic cause of CKD is more common in children than in adults. This study evaluated the diagnostic yield and phenotypic spectrum of children who received genetic testing through the KIDNEYCODE sponsored genetic testing program. Methods: Unrelated children < 18 years of age who received panel testing through the KIDNEYCODE sponsored genetic testing program from September 2019 through August 2021 were included (N = 832). Eligible children met at least one of the following clinician-reported criteria: estimated GFR ≤ 90 ml/min/1.73 m Results: A positive genetic diagnosis was observed in 234 children (28.1%, 95% CI [25.2-31.4%]) in genes associated with Alport syndrome (N = 213), FSGS (N = 9), or other disorders (N = 12). Among children with a family history of kidney disease, 30.8% had a positive genetic diagnosis. Among those with hematuria and a family history of CKD, the genetic diagnostic rate increased to 40.4%. Conclusions: Children with hematuria and a family history of CKD have a high likelihood of being diagnosed with a monogenic cause of kidney disease, identified through KIDNEYCODE panel testing, particularly COL4A variants. Early genetic diagnosis can be valuable in targeting appropriate therapy and identification of other at-risk family members. A higher resolution version of the Graphical abstract is available as Supplementary information.
MeSH term(s)	Adult ; Humans ; Child ; Hematuria/etiology ; Hematuria/genetics ; Glomerulosclerosis, Focal Segmental/complications ; Glomerulosclerosis, Focal Segmental/diagnosis ; Glomerulosclerosis, Focal Segmental/genetics ; Nephritis, Hereditary/complications ; Nephritis, Hereditary/diagnosis ; Nephritis, Hereditary/genetics ; Collagen Type IV/genetics ; Renal Insufficiency, Chronic/diagnosis ; Renal Insufficiency, Chronic/genetics ; Renal Insufficiency, Chronic/complications
Chemical Substances	Collagen Type IV
Language	English
Publishing date	2023-05-19
Publishing country	Germany
Document type	Journal Article
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-023-05993-z
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Article ; Online: The association of low birthweight and prematurity on outcomes in children and adults with nephrotic syndrome-a NEPTUNE cohort study.

Hingorani, Sangeeta / Gibson, Keisha L / Xie, Yuping / Wang, Yujie / Eddy, Sean / Hartman, John / Sampson, Matthew / Cassol, Clarissa / Thomas, David / Gipson, Debbie S / Trachtman, Howard / Srivastava, Tarak / Reidy, Kimberly

Pediatric nephrology (Berlin, Germany)

2023 Volume 38, Issue 10, Page(s) 3297–3308

Abstract: Background: In single-center studies, both preterm birth and low birth weight (LBW) are associated with worse outcomes in childhood nephrotic syndrome. Using the Nephrotic Syndrome Study Network (NEPTUNE) observational cohort, we tested the hypothesis ... ...

Abstract	Background: In single-center studies, both preterm birth and low birth weight (LBW) are associated with worse outcomes in childhood nephrotic syndrome. Using the Nephrotic Syndrome Study Network (NEPTUNE) observational cohort, we tested the hypothesis that in patients with nephrotic syndrome, hypertension, proteinuria status, and disease progression would be more prevalent and more severe in subjects with LBW and prematurity singly or in combination (LBW/prematurity). Methods: Three hundred fifty-nine adults and children with focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD) and available birth history were included. Estimated glomerular filtration rate (eGFR) decline and remission status were primary outcomes, and secondary outcomes were kidney histopathology, kidney gene expression, and urinary biomarkers. Logistic regression was used to identify associations with LBW/prematurity and these outcomes. Results: We did not find an association between LBW/prematurity and remission of proteinuria. However, LBW/prematurity was associated with greater decline in eGFR. This decline in eGFR was partially explained by the association of LBW/prematurity with APOL1 high-risk alleles, but the association remained after adjustment. There were no differences in kidney histopathology or gene expression in the LBW/prematurity group compared to normal birth weight/term birth. Conclusion: LBW and premature babies who develop nephrotic syndrome have a more rapid decline in kidney function. We did not identify clinical or laboratory features that distinguished the groups. Additional studies in larger groups are needed to fully ascertain the effects of (LBW) and prematurity alone or in combination on kidney function in the setting of nephrotic syndrome.
MeSH term(s)	Female ; Humans ; Child ; Infant, Newborn ; Adult ; Nephrotic Syndrome/complications ; Cohort Studies ; Birth Weight ; Neptune ; Premature Birth/epidemiology ; Infant, Low Birth Weight ; Glomerulosclerosis, Focal Segmental/pathology ; Proteinuria/etiology ; Proteinuria/complications ; Apolipoprotein L1/genetics
Chemical Substances	APOL1 protein, human ; Apolipoprotein L1
Language	English
Publishing date	2023-05-04
Publishing country	Germany
Document type	Journal Article
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-023-05876-3
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Article ; Online: Reimagining Nephrology Fellowship Education to Meet the Future Needs of Nephrology: A Report of the American Society of Nephrology Task Force on the Future of Nephrology.

Rosenberg, Mark E / Anderson, Sharon / Farouk, Samira S / Gibson, Keisha L / Hoover, Robert S / Humphreys, Benjamin D / Orlowski, Janis M / Udani, Suneel M / Waitzman, Joshua S / West, Melissa / Ibrahim, Tod

Clinical journal of the American Society of Nephrology : CJASN

2023 Volume 18, Issue 6, Page(s) 816–825

Abstract: The American Society of Nephrology (ASN) Task Force on the Future of Nephrology was established in April 2022 in response to requests from the American Board of Internal Medicine and the Accreditation Council for Graduate Medical Education regarding ... ...

Abstract	The American Society of Nephrology (ASN) Task Force on the Future of Nephrology was established in April 2022 in response to requests from the American Board of Internal Medicine and the Accreditation Council for Graduate Medical Education regarding training requirements in nephrology. Given recent changes in kidney care, ASN also charged the task force with reconsidering all aspects of the specialty's future to ensure that nephrologists are prepared to provide high-quality care for people with kidney diseases. The task force engaged multiple stakeholders to develop 10 recommendations focused on strategies needed to promote: ( 1 ) just, equitable, and high-quality care for people living with kidney diseases; ( 2 ) the value of nephrology as a specialty to nephrologists, the future nephrology workforce, the health care system, the public, and government; and ( 3 ) innovation and personalization of nephrology education across the scope of medical training. This report reviews the process, rationale, and details (the "why" and the "what") of these recommendations. In the future, ASN will summarize the "how" of implementing the final report and its 10 recommendations.
MeSH term(s)	Humans ; United States ; Nephrology/education ; Fellowships and Scholarships ; Education, Medical, Graduate ; Internal Medicine/education ; Nephrologists
Language	English
Publishing date	2023-02-27
Publishing country	United States
Document type	Journal Article
ZDB-ID	2226665-3
ISSN	1555-905X ; 1555-9041
ISSN (online)	1555-905X
ISSN	1555-9041
DOI	10.2215/CJN.0000000000000133
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Zs.A 6584: Show issues

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Article ; Online: Emerging Agents for the Management of Nephrotic Syndrome: Progress to Date.

Gibson, Keisha L / Hansrivijit, Panupong / Ferris, Maria E

Paediatric drugs

2016 Volume 18, Issue 1, Page(s) 25–29

Abstract: Nephrotic Syndrome is a rare condition associated with high morbidity in the 20-40% of children and adolescents who fail to respond to standard immunosuppressive therapies. Novel non-immunologic mechanisms of widely used immunosuppressive therapies, as ... ...

Abstract	Nephrotic Syndrome is a rare condition associated with high morbidity in the 20-40% of children and adolescents who fail to respond to standard immunosuppressive therapies. Novel non-immunologic mechanisms of widely used immunosuppressive therapies, as well as emerging anti-inflammatory drugs, and anti-fibrotics may play a crucial role in the treatment of patients with refractory disease. This article will review some of these treatments and their various stages of investigation.
MeSH term(s)	Adolescent ; Anti-Inflammatory Agents/therapeutic use ; Child ; Humans ; Immunosuppressive Agents/therapeutic use ; Nephrotic Syndrome/drug therapy
Chemical Substances	Anti-Inflammatory Agents ; Immunosuppressive Agents
Language	English
Publishing date	2016-02
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	1492748-2
ISSN	1179-2019 ; 1174-5878
ISSN (online)	1179-2019
ISSN	1174-5878
DOI	10.1007/s40272-015-0148-y
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Zs.A 5205: Show issues

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Article ; Online: Longitudinal analysis of blood pressure and lipids in childhood nephrotic syndrome.

Pediatric nephrology (Berlin, Germany)

2024

Abstract: Background: In the current study, longitudinal BP and lipid measurements were examined in a NEPTUNE cohort of children with newly diagnosed nephrotic syndrome (cNEPTUNE). We hypothesized that hypertensive BP and dyslipidemia would persist in children ... ...

Abstract	Background: In the current study, longitudinal BP and lipid measurements were examined in a NEPTUNE cohort of children with newly diagnosed nephrotic syndrome (cNEPTUNE). We hypothesized that hypertensive BP and dyslipidemia would persist in children with nephrotic syndrome, regardless of steroid treatment response. Methods: A multi-center longitudinal observational analysis of data obtained from children < 19 years of age with new onset nephrotic syndrome enrolled in the Nephrotic Syndrome Study Network (cNEPTUNE) was conducted. BP and lipid data were examined over time stratified by disease activity and steroid exposure. Generalized estimating equation regressions were used to find determinants of hypertensive BP and dyslipidemia. Results: Among 122 children, the prevalence of hypertensive BP at any visit ranged from 17.4% to 57.4%, while dyslipidemia prevalence ranged from 40.0% to 96.2% over a median of 30 months of follow-up. Hypertensive BP was found in 46.2% (116/251) of study visits during active disease compared with 31.0% (84/271) of visits while in remission. Dyslipidemia was present in 88.2% (120/136) of study visits during active disease and in 66.0% (101/153) while in remission. Neither dyslipidemia nor hypertensive BP were significantly different with/without medication exposure (steroids and/or CNI). In regression analysis, male sex and urine protein:creatinine ratio (UPC) were significant determinants of hypertensive BP over time, while eGFR was found to be a determinant of dyslipidemia over time. Conclusions: Results demonstrate persistent hypertensive BPs and unfavorable lipid profiles in the cNEPTUNE cohort regardless of remission status or concurrent steroid or calcineurin inhibitor treatment.
Language	English
Publishing date	2024-02-06
Publishing country	Germany
Document type	Journal Article
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-024-06301-z
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Article ; Online: Diagnosis and Treatment of Patients With Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome: A Delphi Survey.

Floege, Jürgen / Gibson, Keisha L / Praga, Manuel / Radhakrishnan, Jai / Reich, Heather N / Schreuder, Michiel F / Wetzels, Jack F / Tesař, Vladimír / Vivarelli, Marina / Biechele, Steffen / Tonelli, Marcello

Kidney international reports

2022 Volume 7, Issue 9, Page(s) 2081–2085

Language	English
Publishing date	2022-06-23
Publishing country	United States
Document type	Journal Article
ISSN	2468-0249
ISSN (online)	2468-0249
DOI	10.1016/j.ekir.2022.06.010
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Article ; Online: Back to the future: therapies for idiopathic nephrotic syndrome.

Gibson, Keisha L / Glenn, Dorey / Ferris, Maria E

Blood purification

2015 Volume 39, Issue 1-3, Page(s) 105–109

Abstract: Background: Roughly 20-40% of individuals with idiopathic nephrotic syndrome will fail to respond to standard therapies and have a high risk of progression to end stage kidney disease (ESKD). In the last 50 years, no new therapies have been approved ... ...

Abstract	Background: Roughly 20-40% of individuals with idiopathic nephrotic syndrome will fail to respond to standard therapies and have a high risk of progression to end stage kidney disease (ESKD). In the last 50 years, no new therapies have been approved specifically for the treatment of these individuals with recalcitrant disease. Summary: Recent in vitro, translational, and clinical studies have identified novel targets and pathways that not only expand our understanding of the complex pathophysiology of proteinuric disease but also provide an opportunity to challenge the tradition of relying on histologic classification of nephrotic diseases to make treatment decisions. Key messages: The traditional methods of directing the care of individuals with nephrotic syndrome by histological classification or deciding second line therapies on the basis of steroid-responsiveness may soon yield customizing therapies based on our expanding understanding of molecular targets. Important non-immunologic mechanisms of widely used immunosuppressive therapies may be just as important in palliating proteinuric disease as proposed immunologic functions.
MeSH term(s)	Adalimumab/therapeutic use ; Adrenocorticotropic Hormone/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Cortisone/therapeutic use ; Cyclopropanes/therapeutic use ; Histological Techniques ; Humans ; Immunosuppressive Agents/therapeutic use ; Kidney Failure, Chronic/immunology ; Kidney Failure, Chronic/metabolism ; Kidney Failure, Chronic/pathology ; Kidney Failure, Chronic/prevention & control ; Molecular Targeted Therapy ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/immunology ; Nephrotic Syndrome/metabolism ; Nephrotic Syndrome/pathology ; Precision Medicine ; Protein Kinase Inhibitors/therapeutic use ; Pyridines/therapeutic use
Chemical Substances	6-(5-((cyclopropylamino)carbonyl)-3-fluoro-2-methylphenyl)-N-(2,2-dimethylprpyl)-3-pyridinecarboxamide ; Anti-Inflammatory Agents ; Cyclopropanes ; Immunosuppressive Agents ; Protein Kinase Inhibitors ; Pyridines ; Adrenocorticotropic Hormone (9002-60-2) ; Adalimumab (FYS6T7F842) ; Cortisone (V27W9254FZ)
Language	English
Publishing date	2015
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	605548-5
ISSN	1421-9735 ; 0253-5068
ISSN (online)	1421-9735
ISSN	0253-5068
DOI	10.1159/000368951
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Zs.A 1872: Show issues

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Article ; Online: Kidney Injury Molecule-1 and Periostin Urinary Excretion and Tissue Expression Levels and Association with Glomerular Disease Outcomes.

Wu, Qiaoyan / Troost, Jonathan P / Dai, Tiane / Nast, Cynthia / Eddy, Sean / Wei, Boxian / Wang, Ying / Gipson, Debbie S / Dell, Katherine M / Gibson, Keisha L / Kretzler, Matthias / Adler, Sharon

Glomerular diseases

2021 Volume 1, Issue 2, Page(s) 45–59

Abstract: Introductions: Kidney injury molecule-1 (KIM-1) and periostin (POSTN) are proximal and distal tubule injury biomarkers. We tested whether baseline urine KIM-1/creatinine (uKIM-1/cr) and/or uPOSTN/cr correlated with disease severity or improved a ... ...

Abstract	Introductions: Kidney injury molecule-1 (KIM-1) and periostin (POSTN) are proximal and distal tubule injury biomarkers. We tested whether baseline urine KIM-1/creatinine (uKIM-1/cr) and/or uPOSTN/cr correlated with disease severity or improved a remission prediction model. Methods: Baseline uKIM1/cr and uPOSTN/cr were measured on spot urine samples from immunosuppression-free patients enrolled in Nephrotic Syndrome Study Network until December 15, 2014. Urine protein/creatinine (UPCR) and albumin/creatinine (UACR) were measured at baseline, 4 months, and until last follow-up. Glomerular and tubulointerstitial (TI) expression arrays were analyzed from a baseline research renal biopsy core collected during a clinically indicated biopsy.Renal diagnoses were centrally confirmed, sections scanned, and measured morphometrically. Correlations between baseline uKIM-1/cr and uPOSTN/cr and UPCR, UACR, histopathologic features, glomerular and TI KIM-1 and POSTN expression levels, and renal outcomes were assessed. Results: Baseline uKIM-1/cr correlated with UPCR and UACR, and were associated with complete remission after adjustment for proteinuria, histopathologic diagnosis, and treatment. Baseline uKIM-1/cr also correlated with degree of foot process effacement and acute tubular injury. Glomerular and TI KIM-1 expression levels correlated with UPCR and UACR. Higher TI KIM-1 expression levels correlated with interstitial fibrosis, tubular atrophy, and global glomerulosclerosis, while glomerular KIM-1 expression correlated with time to remission. Findings for POSTN were of lesser statistical strength. Discussion/conclusion: Lower baseline uKIM-1/cr values were associated with more rapid time to complete remission after adjusting for proteinuria, histopathologic diagnosis, and treatment. Increased TI KIM-1 expression levels in proteinuric states were associated with chronic morphological injury; lower glomerular expression levels were associated with a greater potential for proteinuria reversibility.
Language	English
Publishing date	2021-03-30
Publishing country	Switzerland
Document type	Journal Article
ISSN	2673-3633
ISSN (online)	2673-3633
DOI	10.1159/000513166
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